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Lily's Story 

Lily had to have corrective surgery for complete AVSD at just six weeks old, I was told afterwards that she still had a leaking valve but was unlikely to need further surgery until her teens. So you can imagine my shock five months later when I was told that Lily needed valve surgery as soon as possible!

I got lots of information from the Cardiac Liaison Nurses and I naively thought after the valve repair everything would be fine. Eventually, after a cancellation, surgery took place when Lily was ten months old. Lily’s surgery lasted over seven hours, after five I was going stir crazy. A surgeon came to tell me that Lily’s valve had disintegrated during surgery and they had no option but to put in a mechanical valve.

No-one knew of a child as young as Lily
who had Down’s Syndrome and a valve replacement

Eventually Lily’s blood became fairly stable and things began to settle down, however, this wasn’t to last. Lily was admitted to the high dependency unit with a severe respiratory infection and a very low platelet count of 8, when it should be 200-400

Lily’s ITP has improved and her platelet count is no longer at a dangerous level but she has dips. Thankfully it is manageable and does not affect her significantly most of the time. Lily’s home INR machine is not accurate enough so this means we have to go to hospital for blood tests at least once a week, or when she is very unstable, every day! Lily has got better at having blood tests as long as they are done in her feet.

Recently we managed six months with no hospital stays! We go on holiday (arranging for blood tests at a local hospital) and do normal activities for a three year old. Lily wears a helmet if she is doing anything really physical, but that is the only outward sign of her medical issues. If you met Lily you would never know what she goes through.

Over the next few days I had a very steep learning curve. I found out about valves, warfarin, INR, blood tests, repeat surgery, blood clots and so on. I contacted various groups including DHG, but no-one knew of a child as young as Lily who had Down’s Syndrome and a valve replacement.

After surgery she progressed in leaps and bounds, managed to get rid of the NG feeding tube she’d had since birth and the overnight oxygen that she hated! She was definitely happier without things stuck to her face and her eating improved rapidly.

She was at major risk of internal bleeds. Lily had intravenous medication for five days which increased her platelet count enough for us to go home, but before the week was out it had dropped rapidly again and we were readmitted.

Between October and December 2010 we were in hospital virtually every other week. Lily was given steroids, immunoglobulins, platelet transfusions, plasma transfusions and more besides. She had a bone marrow aspiration which thankfully was negative for leukaemia. Lily was diagnosed as having ITP (immunothrombocytopoenic purpura) and I was told that it was nothing to do with Down’s Syndrome or her heart, she was just unlucky. .

Due to Lily’s valve replacement she needs to take warfarin to thin her blood. To check how thin blood is, doctors test something called INR. At first Lily’s blood was tested in hospital, later we were given a machine to test her blood at home, reducing trips to hospital. So I learned another new skill – blood testing a wriggly toddler by myself!

Lily’s INR is unfortunately very unstable. We’ve had frequent admissions to hospital; if her INR is too low she has to have intravenous medicine and then blood tests every four hours. If the INR is too high vitamin K is given to reduce it. As a result Lily has become very tactile defensive and will not let anyone touch her hands. She’s developed a fantastic head butt and bite reflex that she aims with great accuracy at the doctors doing the tests! The first time Lily was given vitamin K she had an extreme reaction, resulting in a longer stay in hospital than for the open heart surgery and valve replacement!

She is a happy, sociable and wonderful little girl

I am very proud of Lily and the way in which she takes everything in her stride. She is due to have her valve replaced in the next year and I am hopeful that she will cope with it as well as she has coped with everything else.

Jilly Humphrey

please check out the link to the Downs heart group for great support